One Size Does Not Fit All
Genetic Prediction of Kawasaki Disease Treatment Response in Diverse Populations
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Kawasaki disease (KD) is an acute systemic vasculitis afflicting children predominantly <5 years of age. The KD diagnosis depends on the presence of persistent high fever and clinical symptoms and signs of an inflammatory reaction.1 Although the acute inflammatory phase is usually self-limited, the vasculitis shows a predilection for involving the coronary arteries. Transient or even persistent coronary artery dilation can ensue and result in aneurysm formation. Thrombosis or scarring within the coronary artery wall may eventually lead to clinically important stenosis, often requiring intervention.1 Because of the variable nature of KD, accurate prediction for treatment response during the early phase is essential for successful initial clinical management and treatment.
See Article by Kuo and Wong et al
In this month’s issue of Circulation: Cardiovascular Genetics, Kuo et al2 developed a genetic-based risk score for predicting treatment outcome for KD. Although, the leading cause for acquired heart disease in children from developed nations, the cause for KD remains undefined. Environmental factors likely trigger the autoinflammatory response in genetically susceptible individuals. Japan and other East Asian countries show >10-fold greater KD incidence than North American and European populations.1 The higher incidence persists in East Asians living in the United States,3 suggesting that genetics are responsible, although cultural and dietary factors are also implicated for influencing differences in incidence.4 Accordingly, multiple investigators have pursued hypothesis-derived candidate genes or used genome-wide strategies to identify KD susceptibility loci.1
Intravenous gamma globulin (IVIG) serves globally as the primary treatment for KD. Randomized clinical trials performed in the 1980s suggested that IVIG reduced the prevalence of persistent coronary artery lesions (CAL).5,6 Nevertheless, a more recent clinical trial performed by the National Heart, Lung, and Blood Institute–funded pediatric heart network showed a 19% CAL prevalence rate …